Article
Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency.
The Journal of biological chemistry - 24 Aug 2007
Saheki Takeyori, Iijima Mikio, Li Meng Xian, Kobayashi Keiko, Horiuchi Masahisa, Ushikai Miharu, Okumura Fumihiko, Meng Xiao Jian, Inoue Ituro, Tajima Atsushi, Moriyama Mitsuaki, Eto Kazuhiro, Kadowaki Takashi, Sinasac David S, Tsui Lap-Chee, Tsuji Mihoko, Okano Akira, Kobayashi Tsuyoshi
Abstract excerpt
Citrin is the liver-type mitochondrial aspartate-glutamate carrier that participates in urea, protein, and nucleotide biosynthetic pathways by supplying aspartate from mitochondria to the cytosol. Citrin also plays a role in transporting cytosolic NADH reducing equivalents into mitochondria as a component of the malate-aspartate shuttle. In humans, loss-of-function mutations in the SLC25A13 gene encoding citrin...
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